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132. Kongress der Deutschen Gesellschaft für Chirurgie

Deutsche Gesellschaft für Chirurgie

28.04. - 01.05.2015, München

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CSF leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine

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  • Eric Jose Suero Molina - Universitätsklinikum Münster, Neurochirurgie, Münster, Deutschland
  • Christian Ewelt - Universitätsklinikum Münster, Neurochirurgie, Münster, Deutschland
  • Kushtrim Shala - Marienhospital Borken, Klinik für Allgemein- und Viszeralchirurgie, Borken, Deutschland
  • Walter Stummer - Universitätsklinikum Münster, Neurochirurgie, Münster, Deutschland
  • Jochen Rößler - Universitätsklinikum Freiburg, Pädiatrische Hämatologie und Onkologie, Freiburg, Deutschland

Deutsche Gesellschaft für Chirurgie. 132. Kongress der Deutschen Gesellschaft für Chirurgie. München, 28.04.-01.05.2015. Düsseldorf: German Medical Science GMS Publishing House; 2015. Doc15dgch361

doi: 10.3205/15dgch361, urn:nbn:de:0183-15dgch3611

Published: April 24, 2015

© 2015 Suero Molina et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

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Introduction: Patients with Gorham-Stout disease (GSD), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. Destruction of bone matrix is caused by lymphatic vessels, which can lead to CSF leakage if parts of bony structures adjacent to CSF spaces are involved. So far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with CSF leakage.

Material and methods: We report the case of a 30-year-old man with GSD and CSF leakage due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine. Neurosurgical intervention, including dura repair, was needed. Experimental medical therapy with rapamycin was started, leading to disease control for more than 12 months.

Results: The patient recovered quickly after surgery. Postop- erative MRI demonstrated regression of low-lying cer- ebellar tonsils only 6 mm below the foramen magnum. The patient was discharged from the hospital 14 days after surgery. Rapamycin therapy was started again 4 weeks after surgery. The patient remained in good clini- cal condition without cephalalgia at clinical follow-up 12 months later.

Conclusion: Progression of GSD can lead to destruction of the meninges, causing CSF leakage. We review 4 other cases reported in the literature and discuss therapeutic options.

Figure 1 [Fig. 1]